in the right temporal lobe. His memory seemed to improve slightly. By day 14 he was able to complete the serial sevens test accurately to 58.Complement-fixing antibody to herpes simplex virus (HSV) was found in all serum and CSF specimens (table). For samples taken on days 2 and 14, HSV IgG antibody was quantitated by radioimmunosorbent assay.' Albumin and IgG were determined by electroimmunoassay. The CSF:serum albumin ratio (measuring blood-CSF barrier function) was slightly elevated on day 2 but was within normal limits by day 14. The elevation of CSF:serum albumin ratio was not sufficient to invalidate index measurements.2 Intrathecal IgG synthesis was documented by an elevated IgG index in both specimens. Intrathecal synthesis of HSV-specific IgG antibody was demonstrated in both pairs of specimens by calculation of an HSV-specific antibody index (AI). An A1 of->1.9 is diagnostic of herpes encepha1itis.lTo confirm the diagnosis, CSF from days 2, 14, and 22 were examined for HSV DNA. DNA corresponding to a fragment of the HSV-1 thymidine kinase (TK) gene was specifically amplified using the polymerase chain r e a~t i o n ,~ and the presence of HSV TK gene-specific DNA confirmed by Southern blotting.3 HSV DNA was detected at days 2 and 14, but was absent at day 22.Psychometric assessment carried out 3 weeks after admission and 1 week after cessation of acyclovir therapy showed disturbance of visual and verbal memory functions and, particularly, impaired delayed recall. He could not recall the ages of his children, his address or how long he had been living there, his locality, or details of his employment extending back several years. Retrograde amnesia was estimated as extending for 3 to 4 years. He had marked anterograde amnesia. He was disorientated as to time, unable t o give any account of recent events, and unable to learn new information. He retained insight and there was no confabulation. Personality was intact. Tests of frontal lobe function, language, perception, attention, and concentration were normal, and his full-scale I& was assessed to be in the high average range at 113. A significant feature of his memory disturbance was a positive response to cueing, indicating a deficit of retrieval rather than storage o r encoding of information. He and his wife were given advice in the use of practical

A case of Behr’s disease (complicated optic atrophy) associated with epilepsy and with imbalance of the free amino acid pool in plasma and cerebrospinal fluid, quite similar to that identified in patients with hereditary ataxias, is reported. The latter finding stands for a closer correlation between the two clinical entities.